首个商业化的可用于神经退行性疾病研究的即用型SMN ELISA试剂盒

首个商业化的可用于神经退行性疾病研究的即用型SMN ELISA试剂盒

运动神经元生存蛋白（SMN）是一种约38kDa的蛋白质，主要由位于5q染色体端粒部分的SMN1基因产生。几乎相同的着丝粒拷贝（SMN2）也产生少量的全长SMN蛋白，但由于翻译沉默的C-T转换导致mRNA前体的选择性剪接，所产生的大部分SMN被截断，导致蛋白稳定性降低和整体SMN水平下降。SMN1基因的缺失或突变导致全长SMN蛋白水平降低，在人类中表现为一系列的神经肌肉表型，即脊髓性肌肉萎缩症（SMA）疾病。SMA的特点是肌肉无力、萎缩与功能障碍，是婴幼儿最常见的致命遗传病。大约35个成年人中就有一个是SMN1突变的携带者。活产婴中SMA的发病率为1/6,000至1/10,000。SMN蛋白存在于细胞质中，也存在于细胞核中，它集中在与Cajal体相关的“宝石”结构中。SMN蛋白是含有Gemin的复合物的成分，被认为参与了RNA代谢的许多方面。SMN复合物已被证明可介导富含尿苷的小核糖核酸蛋白（snRNPs）的组装，而snRNPs又充当了剪接体的关键成分。

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产品特点

◆ 高灵敏度，可检测低至50 pg/mL的运动神经元生存蛋白

◆ 完全定量的结果，优于半定量的Western blot分析

◆ 简单易用的预包被板与颜色编码试剂，最大限度地减少实验误差

◆ 高通量，3小时内可得39份样品（含复孔）的检测结果

产品信息

标准曲线示例

部分产品引用文献

A Comparative Study of SMN Protein and mRNA in Blood and Fibroblasts in Patients with Spinal Muscular Atrophy and Healthy Controls: R.I. Wadman, et al.; PLoS One 11, e0167087 (2016)

Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy: M.E. Butchbach, et al.; Exp. Neurol. 279, 13 (2016)

SMN Protein Can Be Reliably Measured in Whole Blood with an Electrochemiluminescence (ECL) Immunoassay: Implications for Clinical Trials: P. Zaworski, et al.; PLoS One 11, e0150640 (2016)

Transcript, methylation and molecular docking analyses of the effects of HDAC inhibitors, SAHA and Dacinostat, on SMN2 expression in fibroblasts of SMA patients: J. Mohseni, et al. ; J. Hum. Genet. 61, 823 (2016)

Spinal Muscular Atrophy Biomarker Measurements from Blood Samples in a Clinical Trial of Valproic Acid in Ambulatory Adults: S.R. Renusch, et al.; J. Neuromuscul. Dis. 2, 119 (2015)

Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy : L.P. Bogdanik, et al.; PNAS 112, E5863 (2015)

A novel evaluation method of survival motor neuron protein as a biomarker of spinal muscular atrophy by imaging flow cytometry: M. Arakawa, et al.; Biochem. Biophys. Res. Commun. 453, 368 (2014)

Severe SMA mice show organ impairment that cannot be rescued by therapy with the HDACi JNJ-26481585: J. Schreml, et al.; Eur. J. Hum. Genet. 21, 643 (2013)

Evaluation of Peripheral Blood Mononuclear Cell Processing and Analysis for Survival Motor Neuron Protein: D.T. Kobayashi, et al.; PLoS One 7, e50763 (2012)

Evaluation of SMN protein, transcript, and copy number in the biomarkers for spinal muscular atrophy (BforSMA) clinical study: T.O. Crawford, et al.; PLoS One 7, e33572 (2012)

Utility of Survival Motor Neuron ELISA for Spinal Muscular Atrophy Clinical and Preclinical Analyses: D.T. Kobayashi, et al.; PLoS One 6, e24269 (2011)